tbuckley/cabot-search huggingface dataset:
DP05 (P53) Incidence of Merkel cell carcinoma in the Republic of Ireland (1994–2019)
Authors: Claire Doyle, Marta Costa Blasco, Orla McFeeley, Paula Beatty, Sarah O’Mahoney, Lisa Murphy, Madonna Andrawis, Anne Marie Tobin
Journal: British Journal of Dermatology (2023)

Abstract Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine cutaneous malignancy most commonly observed in those aged over 65 years. Merkel cell carcinoma is twice as common in males as in females and is 25 times more common in those with fair skin than in those with darker skin types. Solid organ transplant recipients have a 24-fold increased risk of developing MCC. There was a 95% increase in the incidence of MCC in the USA from 2000 to 2013, with further increases predicted. The aim of this study was to investigate the trends of incidence of MCC in the Republic of Ireland. Age-standardized rates were calculated using the 2013 European standard population. There were 447 histologically confirmed MCCs diagnosed between 1994 and 2019. Forty-nine per cent of cases (n = 220) were in males. Median age was 80 years (range 15–19 to 95+). The median age for males was 78 years and the median age for females was 82 years. On average, there were seven cases of MCC diagnosed annually between 1994 and 1998 vs. 27 cases of MCC annually diagnosed between 2015 and 2019—almost a quadrupling of case numbers. The age-adjusted incidence rate of MCC for both male and females is detailed in Table 1. Previous literature has indicated that MCC is twice as common in males than in females. Our cohort differed in that there was a slight female predominance in patients diagnosed with MCC. This is in keeping with published literature from Northern European groups, which have also demonstrated a female predominance in MCC. While the absolute number of MCC diagnosed in Ireland has increased in both males and females, the age-adjusted incidence rate is increasing in males, but there has not been a significant change in females. Other Northern European groups have also demonstrated an increase in the incidence of MCC in males that was not observed in females. This study adds to the body of epidemiological data on MCC. Table 1Age-adjusted incidence rates of Merkel cell carcinoma (MCC)Cases of MCCAge-standardized rate (per 100 000 person-years)SE95% confidence intervalMales 1994–1999230.520.130.27–0.77 2000–2004210.370.080.21–0.53 2005–2009350.790.150.50–1.08 2010–2014661.190.160.88–1.50 2015–2019751.050.120.81–1.29Females 1994–1999260.360.070.22–0.50 2000–2004440.670.100.47–0.87 2005–2009540.790.110.57–1.01 2010–2014450.570.090.39–0.75 2015–2019580.650.090.47–0.83
BH11 Cutis verticis gyrata associated with chronic traction
Authors: Sona Mistry, Matthew Harries, Iskander Chaudhry, Donna Cummins
Journal: British Journal of Dermatology (2023)

Abstract Cutis verticis gyrata (CVG) is a rare condition characterized by overgrowth of the skin of the scalp, leading to skinfolds and deep convoluted furrows that resemble the cerebral cortex gyri. Cases of CVG have been classified into primary essential, primary nonessential and secondary. Primary essential CVG has no associated abnormalities. Primary nonessential CVG is associated with neurological or ophthalmological abnormalities. Secondary CVG is due to a local inflammatory or neoplastic process, or an underlying endocrine or genetic cause. We present two cases of CVG associated with chronic traction alopecia. The main clinical features in both cases were thickened skinfolds in the central scalp, which did not extend to the lateral scalp, recession of the frontal hairline with fringe sign and a negative hair-pull test. Histological findings from the zone of alopecia included nonscarring hair loss with the presence of fibrous tracks within the skin but preservation of the sebaceous glands, consistent with chronic traction alopecia. Both patients were advised to reduce tension on the hair and were treated with topical minoxidil and advised on hair transplantation. Patient 1 was a 15-year-old Sikh boy who developed CVG secondary to chronic traction applied from tying his hair at the top of his scalp when wearing a turban. Owing to cultural practices, the patient could not cut his hair, representing a challenge of treating CVG associated with chronic traction in this group of patients. Patient 2 was a 16-year-old girl with a background of joint hypermobility and recurrent hip dislocations, currently undergoing genetic testing for Ehlers-Danlos syndrome. She developed CVG following chronic traction due to hairstyling. Interestingly, cutting her hair resulted in some improvement of the appearance of CVG, possibly due to reduced hair tension. Chronic traction is an uncommon cause of CVG, with only a few cases reported in the literature. It is likely that the persistent tension of tight hairstyles pulls the scalp into folds and contributes to the appearance of CVG. These cases highlight the importance of ascertaining whether a history of traction styling is present in the diagnosis and management of CVG.
DS10 Down to the bone: bone burring under local anaesthesia for skin cancers involving periosteum at the time of Mohs extirpation — a service evaluation and assessment of patient tolerability
Authors: William Hunt, Katrina Habaluyas, Sarah Fenwick, Dominic Tabor, Stamatina Verykiou, David Brass, Thomas Oliphant
Journal: British Journal of Dermatology (2023)

Abstract We present the initial evaluation of bone burring under local anaesthesia (LA) for skin cancers involving periosteum at the time of Mohs extirpation. Bone burring under LA enables patients to have their procedure on the same day. Including the bone burring as part of the Mohs process is logical as we can most accurately assess the location of the involved periosteal tissue. Our evaluation highlights the evolution of our service, our technique, the cost savings and an assessment of patient tolerability with qualitative data. We use a pneumatic De Soutter microdrill with a rose head drill tip. We have evolved to use a crosshatch pass technique with burring to enable the treated field to have two passes bidirectionally. Bone fragments are gathered and sent for histological and immunohistochemical analysis. Local anaesthesia bone burring is rapid, taking &amp;lt; 5 min. At the time of submission, three patients (aged 78–83 years) with skin cancers were offered the option of bone burring under LA. In each case, the bone appeared clinically normal. All tumours were histologically confirmed infiltrative squamous cell carcinomas on the frontal scalp, nasal dorsum and chin, ranging in size from 30 × 22 to 42 × 30 mm. Mohs stages ranged from two to four, all with periosteal involvement confirmed in the Mohs layer with frozen sectioning. At least a week after the procedure, all patients were asked to rate the pain from the bone burring procedure (0 being no discomfort and 10 being intolerable). The patients all reported a value of 0 (no discomfort). All patients confirmed that they would not have chosen differently between having the procedure done under LA vs. GA. The implementation of LA bone burring not only offers greater convenience to often elderly or frail patients, but also confers a considerable cost-saving. Initial outlay with equipment and maintenance costs are minimal, and staffing costs are modest compared with the general anaesthesia approach, when considering the avoided appointments, dermatology theatre staffing and procedure speed. Bone burring under LA represents an effective, safe, well-tolerated and cost-effective method of achieving clear deep margins in cases of tumour periosteal/bone involvement. Bone burring can be undertaken as part of the Mohs process so that the patient can still have the benefit of a definitive surgical reconstruction on the same day.
P73 Laboratory safety from a 16-week phase III study of dupilumab in patients aged 6 months–5 years with moderate-to-severe atopic dermatitis
Authors: Amy S Paller, Michael J Cork, Andreas Wollenberg, Elaine C Siegfried, Mercedes E Gonzalez, Benjamin Lockshin, Faisal A Khokhar, Zhen Chen, Tayler Gonzalez, Elizabeth Simcox, Randy Prescilla
Journal: British Journal of Dermatology (2023)

Abstract Previous studies of dupilumab treatment in adults, adolescents and children aged 6–11 years with moderate-to-severe atopic dermatitis (AD) showed no clinically meaningful changes in laboratory parameters. Here, we evaluate laboratory safety data for dupilumab-treated children aged 6 months–5 years with moderate-to-severe AD. In LIBERTY AD PRESCHOOL (NCT03346434 part B), a placebo-controlled phase III trial, 162 children aged 6 months–5 years with moderate-to-severe AD were randomized to receive dupilumab 200 mg or 300 mg every 4 weeks (n = 83) based on baseline weight (200 mg: ≥ 5 to &amp;lt; 15 kg; 300 mg: ≥ 15 to &amp;lt; 30 kg), or placebo (n = 79) for 16 weeks. Low-potency topical corticosteroids were initiated from day −14. At baseline, mean (SD) haematology parameters were similar in both treatment groups: eosinophils [dupilumab: 1.1 × 109 cells L−1 (0.7); placebo: 1.1 × 109 cells L−1 (0.7)], platelets [dupilumab: 397.7 × 109 cells L−1 (103.2), placebo: 385.6 × 109 cells L−1 (112.9)] and neutrophils [dupilumab: 3.54 × 109 cells L−1 (1.56); placebo: 3.85 × 109 cells L−1 (1.73)]. Mean (SD) counts of neutrophils [dupilumab: 3.18 × 109 cells L−1 (1.94); placebo: 3.79 × 109 cells L−1 (1.91)] remained within the normal reference interval (RI) at week 16. Mean (SD) change in platelet count at week 16 was −16.3 × 109 cells L−1 (78.5) in the dupilumab group and 17.4 × 109 cells L−1 (106.6) in the placebo group. In the dupilumab group, the mean (SD) change in eosinophil count increased at week 4 [0.48 × 109 cells L−1 (1.8)] and trended downward by week 16 [0.31 × 109 cells L−1 (1.4)], while minimal changes were noted in the placebo group at week 4 [0.1 × 109 cells L−1 (0.7)] and week 16 [−0.2 × 109 cells L−1 (0.7)]. The mean (SD) values for creatine kinase [dupilumab: 144.2 U L−1 (84.8); placebo: 130.7 U L−1 (46.39)], alkaline phosphatase [dupilumab: 273.4 U L−1 (80.01); placebo: 233.2 U L−1 (65.98)] and lactate dehydrogenase [dupilumab: 283.8 U L−1 (44.74); placebo: 317.4 U L−1 (67.58)] at week 16 remained within the normal RI in all treatment groups. Two patients treated with dupilumab reported cases of severe and moderate eosinophilia. Neither event was associated with clinical symptoms nor led to the discontinuation of study treatment. No clinically meaningful changes in haematology or chemistry laboratory safety parameters in children aged 6 months–5 years with moderate-to-severe AD were seen with 16 weeks of dupilumab treatment. These data demonstrate that, as with adults, adolescents and older children, routine laboratory monitoring is unnecessary in this population. Funding sources: research sponsored by Sanofi and Regeneron Pharmaceuticals Inc.
DP26 Syringocystadenocarcinoma papilliferum masquerading as squamous cell carcinoma
Authors: Rona Applewaite, Anisha Mehta
Journal: British Journal of Dermatology (2023)

Abstract Syringocystadenocarcinoma papilliferum (SCACP) is a rare cutaneous adnexal neoplasm with ~30 cases reported. This case highlights the potential diagnostic and treatment challenges. A 52-year-old woman presented with a poorly differentiated carcinoma on the left parietal scalp which recurred 2 and 5 years following the original surgical excision. Initial biopsy of this lesion had shown syringocystadenoma papilliferum (SCAP) with associated syringoma, and a subsequent wider excision revealed suspected poorly differentiated squamous cell carcinoma (SCC) in the deeper dermis that was fully excised; however, the SCAP reached the margins. Excision 2 years later of the first recurrence showed similar histological features, and a second recurrence was excised twice to achieve histological clearance followed by postoperative radiotherapy. She remains tumour-free 2 years later. Upon retrospective review of all specimens, the initial primary tumour excision showed histological findings of a surface exophytic SCAP with mild nuclear enlargement, nucleoli and scattered mitotic figures. An infiltrative lesion was seen beneath this consisting of atypical ductal structures within a fibroblastic stroma. In deeper regions, there was more solid proliferation with some squamous differentiation, although scattered glandular units remained visible. However, the squamous component was predominant in future biopsies and even represented the sole morphological appearance in some. This culminated in the latest biopsy showing a poorly differentiated spindle cell tumour which, with immunohistochemistry (BerEP4-negative; cytokeratin, CK14−, CK5−, p63− and p53-positive; and high Ki67 index), was considered entirely to be a poorly differentiated SCC. Ultimately, this sequence of biopsies actually represented the rare diagnosis of SCACP, which demonstrated progressive squamous differentiation with each recurrence. A previous case published also reported SCACP transition to squamous differentiation (Zhang YH, Wang WL, Rapini RP et al. Syringocystadenocarcinoma papilliferum with transition to areas of squamous differentiation: a case report and review of the literature. Am J Dermatopathol 2012; 34:428–33). Despite the generally favourable prognosis reported, there have been a few cases of locoregional metastasis. Furthermore, recurrence up to 8 years after excision of the primary tumour has been reported (Castillo L, Moreno A, Tardío JC. Syringocystadenocarcinoma papilliferum in situ: report of a case with late recurrence. Am J Dermatopathol 2014; 36:348–52). We present a unique case of doubly recurrent SCACP managed with surgery and adjuvant radiotherapy which highlights the need for long-term follow-up. Furthermore, SCACP may pose a diagnostic challenge due to squamous differentiation of these tumours, which may result in incorrect or delayed diagnosis.
DP04 Role of multidisciplinary team meetings in management of inflammatory skin conditions
Authors: Musfira Shakeel, Amy Foulkes, Richard Warren
Journal: British Journal of Dermatology (2023)

Abstract The multidisciplinary team (MDT) model has been previously demonstrated to improve early diagnosis and outcomes for psoriatic arthritis [Zheng YX, Zheng M. A multidisciplinary team for the diagnosis and management of psoriatic arthritis. Chin Med J (Engl) 2021; 134:1387–9; Luelomo J, Gratacós J, Martínez-Losa MM et al. A report of 4 years of experience of a multidisciplinary unit of psoriasis and psoriatic arthritis. Reumatol Clin 2014; 10:141–6]. However, there is little evidence for a similar role in inflammatory skin disease (ISD). A monthly psoriasis MDT has been established in our tertiary referral dermatology centre. We analysed referrals from February 2021 to February 2022. A total of 37 patient cases were discussed, 29 from within the geographical region of the centre and eight from outside the region. Reasons for referral were the presence of comorbidities; in the majority of cases, this was the presence of an internal malignancy (n = 15). Additional comorbidities were organ transplantation (n = 2), inflammatory bowel disease (n = 2), coexisting inflammatory arthritis (n = 4) and history of severe infection (n = 2). The secondary reason for referral was a history of multiple treatment failures (n = 8) and/or adverse effects of current biologics (n = 2). Where outcome data for regional patients could be assessed, the majority (n = 16) subsequently demonstrated objective improvement in their skin condition, with a mean reduction in Psoriasis Area and Severity Index score of 79.0% (n = 16) and a concurrent mean improvement of 59.6% in Dermatology Quality of Life Index (n = 11). Three patients died (unrelated to psoriasis/treatment). In summary, the presence of comorbidities and prior treatment failures are the primary reasons for referral to a tertiary referral service psoriasis MDT. Multidisciplinary team discussion results in objective improvements in patient care at early follow-up assessments. Further data on MDT outcomes for ISD are required.
P37 An island story: a successful new model of specialist dermatology provision to a rural area
Authors: Kirstin Abraham, Huw Thomas, Alyson Bryden
Journal: British Journal of Dermatology (2023)

Abstract The authors’ health board was invited to take over the dermatology provision of an island health board in 2018. At the point of takeover, the waiting list for new appointments was approximately 33 months. There was a significant burden of return patients, which would take approximately 6 years to process in the previous system. We approached this challenge by implementing several novel strategies. Our own department has an established practice of enhanced vetting of referrals. Essential to this are solid relationships with primary care colleagues to encourage the use of high-quality images within referrals. Referrals are viewed by the vetting dermatology consultant within 48 h. Options following vetting include reassurance regarding benign lesions, or direct treatment advice to the referrer without requiring a clinic appointment. Between August 2018 and December 2022, we were able to deal with 40% of referrals in this way. Other vetting outcomes include direct referral to the local island general surgeons for appropriate lesion removal with advice on excision margins/depth. Pathology results are returned to the dermatologist for interpretation and ongoing care. Vetting consultants can directly book a twice-monthly dermatology video clinic (general or specialist, e.g. paediatric dermatology). Remote monitoring of systemic therapies is undertaken. Dermatology advice is provided for inpatients at the island district general hospital. Suitable patients can be directly booked to see our on-island general practitioner (GP) with a specialist interest in dermatology (GPwSI). We provide quarterly physical visits, consisting of 2 days of consultant-led clinics and complex skin surgery. Our GPwSI colleague is supported by the mainland dermatology team, seeing suitable patients between dermatology visits. They have set up a novel phototherapy service on the islands with support and guidance from the dermatology team. Prior to this, patients needing phototherapy were required to travel to the mainland, often needing prolonged inpatient hospital stays with a significant impact on their jobs, home lives and financial consequences/capacity on the National Health Service. This rural dermatology service is highly effective through the use of enhanced vetting, a well-trained and supported local GPwSI, collaboration with local GPs and general surgeons and remote consulting. The model provides a comprehensive rural service, meaning patients no longer have to travel off island to access general and specialist dermatological services. Using this new model and through dedicated teamworking, there is currently no waiting list for island dermatology patients. Feedback from GPs and patients has been extremely positive.
BI28 Phaeohyphomycosis in the immunosuppressed patients: the challenges of treatment
Authors: Farishta Khan, Sindoora Jayaprakash, Indre Verpetinske
Journal: British Journal of Dermatology (2023)

Abstract Phaeohyphomycosis is a group of multisystem mycoses owed to heterogeneous organisms called ‘dematiaceous’ or ‘melanized’ fungi. The presence of melanin in their cell walls is a distinguishing feature, likely to be acting as a virulence factor. Widely abundant in the environment, these organisms commonly infect humans through traumatic inoculation and have many clinical forms of presentation, including cutaneous, subcutaneous, systemic, and rarely disseminated. Both immunocompetent and immunocompromised hosts are affected. Diagnosis of the cutaneous infection is primarily reliant on histology and culture; treatment is not standardized and is based on clinical experience. We report a case of a 39-year-old man who presented to the dermatology clinic with ‘lumps’ on his lower limbs. His background included poorly controlled hypertension and diabetes mellitus type 2 leading to nephropathy and necessitating a renal transplant, which failed. A 5-year period of dialysis involving a sedentary lifestyle was followed by a repeat renal transplant during which time he developed multiple warty-looking nodules of varying size, with pseudomilia cysts on his lower limbs in addition to stasis changes. Histology reported a pigmented mould infection (probably phaeohyphomycosis), and there was no growth on culture. The patient was trialled on two azole antifungals at different times, but on both occasions, he dived into congestive cardiac failure needing intensive care unit admissions. Reducing immunosuppression therapy was difficult considering his previous failed renal transplant. Owing to the extent and multiplicity of the lesions on the background of immunosuppression, the surgical treatment option poses significant risks. Currently, he is managed on topical treatment with some stabilization of the lesions; however, it is unclear how these will progress in the future. Although uncommon, the number of patients with phaeohyphomycosis has increased due to awareness of melanized fungi on a background of the global COVID-19 pandemic, with SARS-CoV-2, and the use of immunosuppressive agents such as glucocorticoid therapy increasing the risk of infections of deep mycosis. Treatment for phaeohyphomycosis has largely been surgical excision for localized lesions in addition to oral antifungals. Our case highlights that, in transplant patients, therapeutics are difficult, with life-threatening complications from oral antifungals and the risks of surgical intervention including ulceration, bleeding and infections. In addition, the cosmetic appearance of the prevalent noticeable lesions for a relatively young patient is also significant and patient counselling is vital, especially when treatment options are minimal.